UMMS Affiliation

Department of Pediatrics, Division of Hematology Oncology

Publication Date

2018-09-13

Document Type

Article Postprint

Disciplines

Enzymes and Coenzymes | Hematology | Hemic and Lymphatic Diseases | Inorganic Chemicals | Nutritional and Metabolic Diseases | Therapeutics

Abstract

Pyruvate kinase (PK) deficiency is the most common red cell glycolytic enzyme defect causing hereditary non-spherocytic hemolytic anemia. Current treatments are mainly supportive and include red cell transfusions and splenectomy.11 Regular red cell transfusions are known to result in iron overload; however, the prevalence and spectrum of transfusion-independent iron overload in the overall PK deficient population has not been well defined. This analysis describes the prevalence and clinical characteristics of iron overload in patients enrolled in the PK Deficiency Natural History Study (NHS) with a focus on those patients who are not regularly transfused.2

Keywords

Chelation, Iron Overload, Pyruvate Kinase Deficiency, Red Cell Enzyme Abnormalities, Splenectomy

Rights and Permissions

Copyright © 2018, Ferrata Storti Foundation. Authors' accepted manuscript posted as allowed by the publisher's author rights policy at http://www.haematologica.org/information-for-authors.

DOI of Published Version

10.3324/haematol.2018.196295

Source

Haematologica. 2018 Sep 13. pii: haematol.2018.196295. doi: 10.3324/haematol.2018.196295. [Epub ahead of print] Link to article on publisher's site

Journal/Book/Conference Title

Haematologica

Comments

Full author list omitted for brevity. For the full list of authors, see article.

Related Resources

Link to Article in PubMed

PubMed ID

30213831

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